Postural Orthostatic Tachycardia Syndrome (POTS) in hEDS
Postural Orthostatic Tachycardia Syndrome (POTS) is a common and often disabling condition that affects many people with hypermobile Ehlers-Danlos syndrome (hEDS). It causes a rapid increase in heart rate upon standing, leading to symptoms like dizziness, fatigue, and brain fog. Understanding how POTS presents, how it's diagnosed, and how it overlaps with hEDS is essential for proper management and improved quality of life.
Definition and Symptoms of POTS
Postural Orthostatic Tachycardia Syndrome (POTS) is a form of autonomic nervous system dysfunction that causes an abnormal increase in heart rate when standing. It is defined as:
A heart rate increase of ≥30 bpm in adults (or ≥40 bpm in adolescents) within 10 minutes of standing,
without a drop in blood pressure (i.e., no orthostatic hypotension),
accompanied by frequent symptoms that improve when lying down.
Common symptoms include
Lightheadedness or dizziness
Palpitations
Fatigue
Blurred vision
Generalized weakness
Brain fog or cognitive impairment
Exercise intolerance
Tremor
Nausea
Presyncope (feeling faint) or syncope (fainting)
Connection Between hEDS and POTS
Hypermobile Ehlers-Danlos syndrome (hEDS) is a hereditary connective tissue disorder characterized by joint hypermobility, skin hyperextensibility, and tissue fragility. It is frequently associated with dysautonomia, especially POTS. People with hEDS are significantly more likely to experience orthostatic intolerance and autonomic dysfunction than the general population. Up to 80% of people with hEDS report symptoms consistent with dysautonomia, most commonly POTS.
How POTS Is Diagnosed
Medical Diagnosis by a Doctor
POTS is typically diagnosed by a cardiologist or neurologist through clinical history and autonomic testing, which may include
10-minute standing test (active stand test):Measures heart rate and blood pressure changes from lying down to standing.
Tilt table test:A formal evaluation where the patient is tilted upright at a controlled angle while heart rate and blood pressure are monitored.
Holter monitor or event monitor:Tracks heart rate variability over 24–48 hours, especially helpful if symptoms fluctuate.
Diagnosis requires
A sustained heart rate increase of ≥30 bpm (≥40 bpm in adolescents) within 10 minutes of standing or tilt,
in the absence of orthostatic hypotension (no significant blood pressure drop),
along with typical symptoms like dizziness, fatigue, and brain fog.
Doctors may also check for contributing conditions (like anemia, thyroid issues, or low blood volume) and may assess bloodwork, electrolytes, and autonomic reflexes.
Self-Screening and Home Monitoring
People who suspect they may have POTS can try a simplified 10-minute standing test at home
Lie down and rest for 10 minutes; record your heart rate.
Stand up (unsupported) and record your heart rate at 1, 3, 5, 7, and 10 minutes.
A heart rate increase of ≥30 bpm (≥40 bpm in adolescents) with typical symptoms may suggest POTS.
Important: Self-tests are not a substitute for medical diagnosis. If results are suggestive, see a healthcare provider for formal testing and evaluation.
Theoretical Mechanisms Linking hEDS to POTS
Though no single cause explains the overlap, several biological theories help describe why hEDS may lead to POTS:
Connective tissue laxity in veins may cause blood pooling in the lower body when standing, triggering a rapid increase in heart rate to compensate.
Small fiber neuropathy, affecting the tiny nerves that regulate blood vessel tone and sweat production, may impair vasoconstriction.
Low blood volume (hypovolemia) is often present and may reduce blood flow to the brain.
Sympathetic neurogenic dysfunction (impaired nerve signaling) and baroreflex hypersensitivity (abnormal blood pressure regulation) have also been implicated.
Deconditioning from chronic fatigue, pain, or joint instability may worsen autonomic instability.
Treatment Options
Treatment typically begins with non-medication strategies, and medications are added only if symptoms persist or are disabling.
Non-pharmacologic interventions
Fluids: Aim for 2–3 liters of water per day.
Salt: Up to 10–12 grams/day, if tolerated.
Compression garments: Especially full-length, waist-high options.
Exercise: Graded and consistent reconditioning, starting with horizontal (recumbent bike, rowing, swimming) and progressing slowly to upright activities.
Avoid triggers: Heat, prolonged standing, and large meals can worsen symptoms.
Medications (if needed)
Low-dose beta-blockers (e.g., propranolol 10–20 mg): To reduce heart rate.
Fludrocortisone: Increases fluid retention and blood volume.
Midodrine: Constricts blood vessels and raises blood pressure.
Others: Ivabradine, pyridostigmine, or selective medications depending on symptom profile.
Treatment should be tailored to each individual’s underlying physiology and symptom burden, as recommended by the Heart Rhythm Society.
Special Considerations for hEDS Patients
Because hEDS affects joints, connective tissue, and other systems, POTS treatment must be adjusted accordingly.
Exercise modification: Physical activity is essential for recovery, but it must be adapted to prevent joint injuries. Programs should focus on low-impact, progressive, and supervised exercises tailored to individual joint stability and energy levels.
Pain and fatigue management: Chronic musculoskeletal pain, fatigue, and proprioception deficits may limit participation in standard exercise regimens.
Gastrointestinal concerns: Many people with hEDS have gastroparesis, IBS, or food intolerances, which can make high salt intake difficult. Dietitian support may be needed.
Mast cell activation syndrome (MCAS): This common comorbidity may interfere with medication tolerance and contribute to systemic inflammation and autonomic dysfunction.
Medication sensitivities: People with hEDS often have complex responses to medications and may require lower starting doses and close monitoring.
Summary
POTS is a common form of dysautonomia in people with hEDS, often triggered by poor blood return to the heart when standing due to connective tissue laxity.
Symptoms include dizziness, fatigue, rapid heart rate, brain fog, and fainting, and often improve when lying down.
POTS is diagnosed through clinical history, standing tests, tilt-table testing, and other autonomic evaluations, with self-screening as an initial step.
Treatment combines hydration, salt, compression, exercise, and medications, tailored to the individual.
People with hEDS need special adjustments due to fragile joints, comorbid GI issues, and potential medication sensitivities.
